Objective To explore the clinical features of patients with end-stage renal disease (ESRD) complicated with pulmonary tuberculosis (PTB) and extrapulmonary tuberculosis (EPTB) and undergoing maintenance hemodialysis (MHD). Methods A total of 133 ESRD patients complicated with tuberculosis, treated at the Tuberculosis Department of the 8th Medical Center of Chinese PLA General Hospital and undergoing MHD at the Blood Purification Center, were selected from July 2012 to February 2021, and divided into PTB group (87 cases) and EPTB group (46 cases). The clinical characteristics, treatment and prognosis of the two groups were compared and analyzed. Results Both PTB group and EPTB group were predominantly male patients (80.5% and 58.7%), but the proportion of female patients was higher in EPTB group (41.3%) than that in PTB group (19.5%, P<0.05). No significant differences existed between the two groups in age, primary kidney disease, comorbidities, dialysis duration, and the detection time-point of tuberculosis after dialysis. Among the first symptoms, the proportion of cough+sputum and fever was significantly higher in PTB group than that in EPTB group (59.8% vs. 23.9% and 60.9% vs. 28.3%, respectively, P<0.05), while the proportion of pain at lesion location was significantly lower in PTB group than that in EPTB group (8.0% vs. 41.3%, P<0.05).CT or MRI imaging was the dominated method for definite diagnosis in both PTB group and EPTB group (67.8% and 76.1%), the secondary method was sputum smear or culture (26.4% and 6.5%) and needle biopsy (5.7% and 17.4%), respectively, with a significant difference between the two groups (P<0.05). No significant difference existed in the positive rate of γ-interferon release assay (γ-IRA) between the two groups. In both PTB group and EPTB group, the proportion of lymphocytes, hemoglobin and albumin decreased markedly, while the proportion of erythrocyte sedimentation rate (ESR) and increased C-reactive protein(CRP) were relatively higher, but there was no statistical difference between the two groups. Both groups were treated with anti-tuberculosis drugs, and one case in EPTB group received anti-tuberculosis drugs plus surgery of tuberculosis focus clearance. No significant difference existed in the incidence of adverse reactions between the two groups, and the improvement rate after treatment was similar. Conclusions Significant differences exist between PTB and EPTB patients with ESRD on MHD in gender ratio, first manifested symptom and diagnosis methods. Paying attention to these differences and conducting individualized monitoring, diagnosis and treatment will be helpful to reduce the morbidity and mortality of patients with PTB and EPTB.